My Life with Lymphedema and Lymphoma

OK…so a dear friend wrote me and said, “the blog needs to be more about the person then lymphedema.” …so I guess that is what a blog is for.

I very much want to put in an article on coping and adjusting, but first, let me share a bit about what it has been like to have the combination of hereditarylymphedema and secondary lymphoma.

In my first post, I shared some of the initial experience, so in this one, I'll just bring everything up to date. My experience, to say the least, has been one of total frustration. The ignorance of the medical community regarding lymphedema is bad enough…but throw in secondary lymphoma, and it becomes even more complicated.

Yet, I also do have to be honest and say that along the way, I have been blessed with having three doctors who have made the difference and for whom I have a deep admiration and respect. Not only are they brilliant in their medical skills, but deeply care about and listen to their patients. They are Dr. Hamilton Williams III, Radiology Oncologist; Elliott Raizes, Infections; and now Stephen Szabo, Oncologist (specializing in lymphoma). I share more about them later.

Fortunately, secondary lymphoma is rare with lymphedema. But when it does occur, it is almost always Cutaneous T-cell lymphoma. Mine, however, are B-cell lymphomas. The first one, diagnosed in 1995, was mixed B-cell and the second, diagnosed in 2000, was lymphoplasmacytic, another B-cell type. Dr. Peter Mortimer, a renown lymphedema doctor and researcher from the UK, told me earlier this year I was only the second case he had heard of in his career.

So, I have been charting new territory. …I love the thought of going where no one has gone before, just never thought it would be with lymphedema and lymphoma. With odds like this….how come I never win the lottery???

When I was first diagnosed, the newer monoclonal antibody drugs like Rituxan were not yet available. Standard chemo was ruled out because of my immune system and the concern over what the chemo would do to the extensively fibrotic tissue of the leg.

While it is a given that radiation is not good for a fibrotic lymphedema limb, that was the only option we had at the time to try any type of treatment. Dr. Williams designed a treatment program where I would receive radiation at a lower then normal dose over a longer period of time. This was effective against the initial tumor, but, of course, was not able to treat any cancer cells elsewhere.

In the meantime, I went through several oncologists trying to find one that might want to have a clue on how to help. I was finally referred to this oncologist who was a former professor of oncology at Georgia's premier medical school. What a total and complete waste of time. His ignorance was matched only by his indifference and callous attitude. I subsequently just walked away from any contact with cancer doctors. Also, I found out recently he had reacted the same way to other patients who had developed secondary lymphedema from their cancer.

My infections doctor, Dr. Elliott Raizes, had long been after me to see this oncologist he knew. Since things became so terrible for me this year, felt like maybe I needed to give him a shot.

Well, couldn't be any more impressed or happier than I am with him. Dr. Szabo is proving to be just as much as a life saver as Dr. Raizes.

After all the tests were done, he decided on a treatment program of four (4) week sessions of a drug called Rituxan. This wasn't developed when I first had lymphoma and he was very very surprised that the other oncologist had not tried it sooner, especially considering the positive biopsies and subsequent complications that I have been experiencing.

The first session went well. Only odd experience was that when the Rituxan first hit my legs I had a horrible reaction of massive itching and the color turned a dark purple. The treatment nurse quickly followed up with more injections of Benadryl that helped calm things down. Fortunately, this did not occur during the next three sessions.

We saw immediate positive reaction to the Rituxan. This wonder drug is so target-specific that if something reacts to it, you can be sure it was a B-cell malignancy. Dr. Szabo felt that there was extensive infiltration in the skin and this made sense due to the plaque and condition of the skin in the left leg.

The plaque shrank (actually almost like withered) and a nickel-size tumor on the back of the leg shrank considerably.

So now…a month past treatment…only a couple downers.

Just going through my first post-chemo infection. Hopefully, the oral Bactrim will handle it and I won't have to go back on IV. Secondly, that tumor has again started growing…seemingly faster then it had before.

I have a followup in a couple weeks and we will go from there.

But all in all, I am more optimistic now than I have been in a long time.
In Part Two - I'll share some clinical considerations about B-cell lymphoma and primary lymphedema. God forbid any visitor/reader should experience this, but if you do, at least this information will be available to you.

Clinical Considerations for the Diagnosis and Management of Lymphoma with Primary Lymphedema

The key to understanding lymphoma as a secondary condition to primary lymphedema is in the pathophysiology of the lymph system.

Introduction

In the situation of primary lymphedema, the lymph system is either incompletely formed or malformed. This means that the spread of and the expression of lymphoma (course of the disease) is going to be different than in an individual with a “normal” lymph system.

The reason is simple. With the lymphatic flow constricted, the malignancy will have a more difficult time spreading to external nodal areas. The incomplete lymph system will act as a “dam” actually helping to contain the lymphoma.

Because of either the hypoplasia or hyperplasia of the lymphatics, it is going to be more difficult to obtain an accurate diagnosis of a lymphatic cancer.

Point One

The nodes of a person with lymphedema may already be smaller than normal. Therefore, if there is limited nodal enlargment (or even if there is none) you cannot assume no malignancy is present.

Furthermore, I have yet to experience really significant lymph node enlargement. But, what I have experienced are nodes that are only slightly larger than “normal,” yet have turned hard and rubbery.

Point Two

Standard radiological tests must be viewed carefully in using them to chart lymphatic cancer with lymphedema. MRIs and CATs are of limited use as the “normal” size node they are picking up may, in fact, be malignant.

Case in point: When the small needle biopsy of my right inguinal lymph node was performed, the doctor did an ultrasound first. That node looked perfectly normal to the ultrasound. The biopsy came back positive.

Point Three

The use of a PET scan in the staging and diagnosis must be viewed with skepticism. There are important reasons for this. The principle behind the PET is quite simple. Tumor (malignancies) will have a higher metabolic rate then the surrounding nonmalignant tissues. Therefore, the malignancies will have a higher “uptake” of the contrast used.

Because of the dysfuntion of the lymph system, it is going to be more difficult for the contrast to make its way through the body and be absorbed. This will affect the diagnostic effectiveness.

Unfortunately too, there is also no standard uptake value for tissue effected by lymphedema. Therefore, it is going to be difficult to ascertain whether an SUV number indicates a malignancy or not.

On the opposite side, lymphedema patients are faced with constant inflammation and/or chronic low-grade infection. Both of these can give a false positive on a PET scan.

Point Four

After the diagnosis of a lymphatic cancer is achieved, the treating physician must carefully monitor changes in the complications experienced by the patient. New complications and changes must not automatically be assumed to be caused by lymphedema.

Case in point: Before the spread and/or infiltration of lymphoma throughout my system, I did not have lung fluid. Two years ago, I began having fluid accumulate in my right lung. This year, the left lung has also been extensively involved.

Also, my lymphedema has been consistent for decades. However, during the past two years, I have experienced swelling of my left arm for the first time.

It is important to understand that those of use with a dysfunctional lymph system walk a fine line. In my situation, with an already at-risk lymph system, the lymphoma spreading through the chest was enough to “overload” the impaired system resulting in both pleural effusions and swelling of the left arm.

Perhaps the most important point to make is for the physician to “listen” to the patient. Individuals like myself have lived with lymphedema for decades - most for years.

We know how lymphedema effects our bodies, what is (normal) for ourselves and what should or should not be happening.

Listen…care about…communicate with your patient.

Lymphoma and Lymphedema - An Update

It has been a while since I put an update on my lymphedema and lymphoma, so thought I would share what has been happening.

I went through four weeks of Rituxan chemo in August/September. I experienced immediate results and a tumor on the lower back calf actually shrank and started to look like it was caving in on itself.

The results were short-lived, however, and the tumor robustly sprouted back to life…seemingly growing faster then it had before.

So, I was off to have a “punch” biopsy. The results took forever and getting an actual diagnosis was even more complicated. This was a result of possible inflammation and the fact that this tumor had already been hit with chemo.

But, what it showed was more than interesting and has caused more questions then it provided answers.

The B-cell lymphoma is still definitely there and apparently active. But the results also showed that about 40 percent of the cell population of the tumor was comprised of “T” cells that also came back with a positive stain for CD3. Is this because of possible inflammation…or are we witnessing the birth of yet another lymphoma? Possible T-cell lymphoma? We honestly can't say and don't know.

Another very interesting report was on the skin itself. My lymphedema is caused by “hypoplasia” of the lymphatics. This means the lymphatic system is constricted and/or missing important parts. Tests done when I was a teen showed that I am missing significant lymph nodes in the inguinal regions. However, the pathologist reported lymphangiectasia of the skin. This means that the lymphatics of the skin are actually dilated instead of constricted.

I had never heard of this with primary/hereditary lymphedema. But, after much research, I have found this is not so unusual with lymphedema as long standing as mine.

The deep lymphatics are constricted, while the superficial lymphatics are dialated–both cause the lymph system to be inefficient and unable to move fluids properly–which, of course, adds to the leg swelling.

I had another round of Rituxan on December 2nd. Kind of disappointed as this time that tumor has not even slowed down. Not only has it continued to grow, but the area surrounding it has changed significantly too.

After 53 years of lymphedema, I am still learning and still finding out new things about this condition.

April 30, 2006 Part Three

Since my last update in December, much has happened in my ongoing struggle with lymphoma and lymphedema.

I have continued the Rituxan treatments and hopefully that is helping blunt and/or remove the lymphoma. This will be continued every three weeks for the next two years.

There has, however, been a dramatic change in the lymphedema. For the last couple months, I have experienced significant over-all body lymphedema. This includes incredible difficulty in breathing, significant new swelling now in both arms, abdomen, chest, and even back.

For many years, I tried to get my former oncologist to understand that B-cell lymphoma with hereditary lymphedema would not follow the same progression path as lymphoma by itself.

As all this new lymphedema has occured, I have wondered how much of it is lymphoma…how much is just the lymphedema.

I ran across, quite by accident today, the first clinical report I have been able to locate on B-cell lymphoma and its overall effect on the lymph system itself. For me it is stunning, as it is verifiable proof of what I have been saying for years.

Lymphatic vasculature in solid tumors may serve as the pathway for metastatic spread of the cancer to the regional lymph nodes and to distant organs. Controversy still exists whether tumors metastasize through existing lymphatics or through newly formed vessels (lymphangiogenesis). The role of lymphangiogenesis in lymphoma spread and proliferation is not clearly established.

VEGF-C is the most potent inducer of lymphangiogenesis. LYVE-1 was shown to be a specific marker for lymphatic vessels in normal and tumor tissue. The aim of the present study was the evaluation of lymph node LYVE-1-positive lymphatic sinus density (LSD) and VEGF-C expression in patients with non-Hodgkin's lymphoma (nHL) and in reactive lymph nodes. Sixty paraffin-embedded lymph nodes from newly diagnosed patients with B-cell nHL were evaluated. Twelve lymph node biopsy specimens from adult patients with reactive lymphonodulitis were used as controls. Sections of lymph nodes were stained immunohistochemically for LYVE-1 and VEGF-C.

VEGF-C expression in lymph nodes of nHL patients was low and not significantly different from that in the control (p = 0.6). Moreover, VEGF-C expression did not differ significantly between aggressive and indolent lymphomas (p = 0.53). Similarly we did not find differences in LSD in aggressive nHL and in indolent nHL (p=0.49). The mean LSD in reactive lymph nodes was higher than in nHL (p = 0.03). Only in 2 out of 12 reactive lymph nodes LYVE-1-positive vessels were absent.

In all groups we demonstrated a strong positive correlation between VEGF-C and LYVE-1-expression (p = 0.0001). Higher LSD in reactive lymph nodes as compared to those of nHL patients suggests that lymphoma proliferation leads to the destruction of the existing lymphatics rather than to lymphangiogenesis within lymph nodes. NHL are not associated with increased expression of VEGF-C nor increased LYVE-1-positive lymphatic sinuses density within lymph nodes.

My Life with Lymphedema and Lymphoma - January 2007

It has been a very long time since I posted on how my life is with lymphedema and lymphoma, so I thought I would put in a brief update.

Something started going terribly wrong with my body in early 2006 and I began to experience total body lymphedema. My abdomen and chest filled up with fluids and I was not able to stop or prevent it from becoming worse.

It was truly one of the most horrible experiences of my life as I became more and more incapacitated and the pain associated with it was overwhelming, both physically and psychologically.

I became unable to do even the most simple of tasks like tying my shoes and even eventually could no longer take care of my legs by putting lotion on them and bandage wrapping them.

By the time of the National Lymphedema Network's conference in very early November, I was barely able to get around with a cane. At the conference, I was confined to a wheelchair for a large portion of the conference. I honestly could not have made it through the conference without the help of Joan White and Cheri Hoskins who were always there to help and to push me from event to event. A therapist named Carmelita Rifkin also came to my room each day to wrap and care for my legs. All three were truly angels of kindness and I will always have a deep, deep appreciation for all they did.

The conference ended and I returned to work, but for only a week. By November 12th, I was so full of fluids that I could breathe only while standing up. My son, Patrick, took me to the emergency room at Gwinnett Medical Center and I was subsequently admitted as an inpatient.

I was in that hospital from November 12, 2006, to December 8, 2006, when I was transferred by ambulance to the Lakeshore Rehabilitation Hospital in Birmingham, Alabama, to be under the care of Dr. Paula Stewart.

All in all, the doctors were able to remove around 60 pounds of fluids from my chest and abdomen. During the first week at Gwinnett, they also drained two and a half quarts of fluid from the right lung. Interestingly, the fluid has always previously been a golden amber color, this time it was pure chylous…milky white in color. On December 22, 2006, I was finally released from Lakeshore and came home.

Then, on the afternoon of December 27th, I began to experience horrendous pain in my back between the left shoulder blade and spine. Again, I was taken to Gwinnett and admitted with a pnuemothorax. They again drained my right lung. In only five weeks, two quarts of fluid had returned to the lung.

I was released on December 31st and finally returned to work on January 4, 2007.

But something had to be done about the lung fluid as you can not safely keep going back time after time to drain the lung, so a pulmonary doctor and a thoracic surgeon was called in.

This coming Monday, January 15th, I will be admitted, yet again, to Gwinnett and will undergo a surgical procedure. Several small incisions will be made and the area between the lung lining and the rib lining will be filled with talcum powder in hopes that it will effectively block off the cavity from further fluid accumulation.

While the doctor has said there may be only a 50-50 chance of success due to the lymphedema, I feel it is a chance I must take. Each month now, it seems that right lung fills up with a couple liters of fluid. There are only so many times that you can drain a lung before complications set in.

The worst part and perhaps the most painful is this tube that will be sticking out of my side for a few days. While he is in there, a small camera will be inserted and some biopsies of the pleura will be taken. If all goes well, he will drain the left lung too, as it shows about one and a half liters of fluid.

The bad thing is that no one really has any clear explanation of what triggered all these events. It was also discovered that my blood protein level had dropped to a dismal 1.5, which is far, far short of the ideal 4.5-5.5 level.

Dr. Stewart has one theory that ties the drop in the protein level in with the lymphedema to cause all the swelling and fluid collection.

The other theory involves the lymphoma. My incredible oncologist, Dr. Stephen Szabo would describe the lymphoma as “not in remission but contained.” So somehow perhaps everything just went together to cause this disaster.

I'm just so relieved though that thus far, the massive swelling has not returned and that I am once again able to do so many things…especially play with my new grandson Connor, who was born on October 15, 2006.

Additional update soon;-)
I promise:-D

See also: My Life with Lymphedema REMEMBER - I started a blog by the same name some five years ago and it should not be confused with any other site that uses that name in hopes of attracting followers. If it doesn't say Pat O'Connor, it isn't the real thing. Blog - My Life with Lymphedema